Multiclinic Observations on the Simplified Diet in PKU

Bernstein, Laurie; Burns, Casey; Sailer-Hammons, Melissa; Kurtz, Angela; Rohr, Frances J.

doi:10.1155/2017/4083293

Cited by 13 publications

(15 citation statements)

References 15 publications

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“…It is acknowledged that internationally, different systems are used to calculate/measure protein/phenylalanine in the PKU diet, each with its own inherent weaknesses [4][5][6][7][8][9]. Using upper protein/phenylalanine cut-off points has the disadvantage of having to measure/calculate foods as part of an exchange system if they contain protein marginally over the cut-off, whilst eating foods as exchange-free if they are just under the cut-off point, but it does give direct guidance and allows many foods to be eaten without measurement or calculation.…”

Section: Discussionmentioning

confidence: 99%

“…Whilst the goal of dietetic management is essentially the same, the method for allocating phenylalanine/protein intake varies between and sometimes within countries, with insufficient evidence to recommend any one method [4][5][6][7][8][9]. Broadly there are two different methods, each with its merits and drawbacks: (1) patients are allocated a daily amount of phenylalanine/protein from all food, which is calculated to provide the prescribed phenylalanine intake; and (2) a phenylalanine/protein exchange system whereby amounts of food are calculated for a defined amount of phenylalanine (ranging from 10-50 mg of phenylalanine for each exchange) or protein (e.g., 1 g protein = 1 exchange).…”

Section: Introductionmentioning

confidence: 99%

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Uniformity of Food Protein Interpretation Amongst Dietitians for Patients with Phenylketonuria (PKU): 2020 UK National Consensus Statements

et al. 2020

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In phenylketonuria (PKU), variable dietary advice provided by health professionals and social media leads to uncertainty for patients/caregivers reliant on accurate, evidence based dietary information. Over four years, 112 consensus statements concerning the allocation of foods in a low phenylalanine diet for PKU were developed by the British Inherited Metabolic Disease Dietitians Group (BIMDG-DG) from 34 PKU treatment centres, utilising 10 rounds of Delphi consultation to gain a majority (≥75%) decision. A mean of 29 UK dietitians (range: 18–40) and 18 treatment centres (range: 13–23) contributed in each round. Statements encompassed all foods/food groups divided into four categories based on defined protein/phenylalanine content: (1) foods high in protein/phenylalanine (best avoided); (2) foods allowed without restriction including fruit/vegetables containing phenylalanine ≤75 mg/100 g and most foods containing protein ≤0.5 g/100 g; (3) foods that should be calculated/weighed as an exchange food if they contain protein exchange ingredients (categorized into foods with a protein content of: >0.1 g/100 g (milk/plant milks only), >0.5 g/100 g (bread/pasta/cereal/flours), >1 g/100 g (cook-in/table-top sauces/dressings), >1.5 g/100 g (soya sauces)); and (4) fruit/vegetables containing phenylalanine >75 mg/100 g allocated as part of the protein/phenylalanine exchange system. These statements have been endorsed and translated into practical dietary management advice by the medical advisory dietitians for the National Society for PKU (NSPKU).

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Uniformity of Food Protein Interpretation Amongst Dietitians for Patients with Phenylketonuria (PKU): 2020 UK National Consensus Statements

et al. 2020

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“…Some centres introduced lower Phe containing fruits and vegetables at weaning without calculation which should benefit infants by improving infant exposure to a wider range of tastes and textures. Over recent years the system of allocating dietary Phe has provoked much discussion but increasing evidence is supporting a ‘simple’ diet in PKU [[12], [13], [14]] and there is no data to suggest that this is associated with loss of blood Phe control. The European PKU Guidelines [15] supported a system where fruits and vegetables containing Phe ≤75 mg/100 g are considered as ‘exchange free’ foods and thereby permitted without measurement [[16], [17], [18]].…”

Section: Discussionmentioning

confidence: 99%

Weaning practices in phenylketonuria vary between health professionals in Europe

Pinto¹,

Adams

Ahring

et al. 2019

Molecular Genetics and Metabolism Reports

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BackgroundIn phenylketonuria (PKU), weaning is considered more challenging when compared to feeding healthy infants. The primary aim of weaning is to gradually replace natural protein from breast milk or standard infant formula with solids containing equivalent phenylalanine (Phe). In addition, a Phe-free second stage L-amino acid supplement is usually recommended from around 6 months to replace Phe-free infant formula. Our aim was to assess different weaning approaches used by health professionals across Europe.MethodsA cross sectional questionnaire (survey monkey®) composed of 31 multiple and single choice questions was sent to European colleagues caring for inherited metabolic disorders (IMD). Centres were grouped into geographical regions for analysis.ResultsWeaning started at 17–26 weeks in 85% (n = 81/95) of centres, >26 weeks in 12% (n = 11/95) and < 17 weeks in 3% (n = 3/95). Infant's showing an interest in solid foods, and their age, were important determinant factors influencing weaning commencement. 51% (n = 48/95) of centres introduced Phe containing foods at 17–26 weeks and 48% (n = 46/95) at >26 weeks. First solids were mainly low Phe vegetables (59%, n = 56/95) and fruit (34%, n = 32/95).A Phe exchange system to allocate dietary Phe was used by 52% (n = 49/95) of centres predominantly from Northern and Southern Europe and 48% (n = 46/95) calculated most Phe containing food sources (all centres in Eastern Europe and the majority from Germany and Austria). Some centres used a combination of both methods.A second stage Phe-free L-amino acid supplement containing a higher protein equivalent was introduced by 41% (n = 39/95) of centres at infant age 26–36 weeks (mainly from Germany, Austria, Northern and Eastern Europe) and 37% (n = 35/95) at infant age > 1y mainly from Southern Europe. 53% (n = 50/95) of centres recommended a second stage Phe-free L-amino acid supplement in a spoonable or semi-solid form.ConclusionsWeaning strategies vary throughout European PKU centres. There is evidence to suggest that different infant weaning strategies may influence longer term adherence to the PKU diet or acceptance of Phe-free L-amino acid supplements; rendering prospective long-term studies important. It is essential to identify an effective weaning strategy that reduces caregiver burden but is associated with acceptable dietary adherence and optimal infant feeding development.

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“…The Simplified Diet is an approach to PKU dietary management that allows for unrestricted intake of foods low in Phe, including many fruits, vegetables, and modified low‐protein medical foods; these foods are considered “free” or “uncounted.” While the diet is simplified, it is not liberalized in terms of increasing the overall amount of Phe that is allowed; rather, the amount of “counted” Phe allowed in the diet is decreased to offset the small amount of Phe in free/uncounted foods. Typically, an individual's dietary Phe prescription is decreased by approximately 30% to account for intake of Phe from free/uncounted foods, and all other foods with higher protein content continue to be measured and counted towards the daily Phe goal . The Simplified Diet may promote increased diet flexibility and encourage greater intake of very low‐Phe foods, such as fruits and vegetables, by removing the patient burden of tracking free/uncounted foods .…”

Section: Introductionmentioning

confidence: 99%

“…The Simplified Diet may promote increased diet flexibility and encourage greater intake of very low‐Phe foods, such as fruits and vegetables, by removing the patient burden of tracking free/uncounted foods . The Simplified Diet has been shown to maintain comparable metabolic control compared to patients following a traditional diet where all foods are counted and measured …”

Section: Introductionmentioning

confidence: 99%

Simplified Diet for nutrition management of phenylketonuria: A survey of U.S. metabolic dietitians

et al. 2020

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Background Phenylketonuria (PKU) is an inherited metabolic disorder affecting the conversion of phenylalanine (Phe) to tyrosine. Medical nutrition therapy, consisting of a Phe‐restricted diet with medical formula, is the primary treatment for PKU. The Simplified Diet is an approach to PKU nutrition management that allows certain fruits, vegetables, and low‐protein foods to be eaten without measuring or tracking, referred to as free/uncounted foods. There is no consensus on how to implement this approach in metabolic centers in the United States (U.S.), and clinical practice varies. Aim This study describes the clinical experience of metabolic dietitians in U.S.‐based metabolic centers related to the use and implementation of the Simplified Diet. Methods A survey was developed and sent out to metabolic dietitians to query current clinical practices related to the Simplified Diet. Descriptive statistics were used to analyze responses. Results Sixty‐three dietitians managing ≥5 patients with PKU in U.S.‐based metabolic centers responded to the survey. Ninety‐eight percent of survey respondents reported using some version of the Simplified Diet in clinical practice. The survey identified areas of strong agreement, including introduction of the Simplified Diet at 6 to 12 months of age. The survey also identified areas of widespread variability, including specific Phe or protein thresholds for free/uncounted foods, and whether or not to set daily quantity limits on these foods. Conclusions Significant variability related to implementation of the Simplified Diet exists across U.S.‐based metabolic centers. This practice variability may contribute to differences in the patient experience across centers and may indicate a need for development of clinical guidelines.

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Multiclinic Observations on the Simplified Diet in PKU

Cited by 13 publications

References 15 publications

Uniformity of Food Protein Interpretation Amongst Dietitians for Patients with Phenylketonuria (PKU): 2020 UK National Consensus Statements

Uniformity of Food Protein Interpretation Amongst Dietitians for Patients with Phenylketonuria (PKU): 2020 UK National Consensus Statements

Weaning practices in phenylketonuria vary between health professionals in Europe

Simplified Diet for nutrition management of phenylketonuria: A survey of U.S. metabolic dietitians

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